Classification, presentation, and initial treatment of Wegener's granulomatosis in childhood

David A. Cabral, América G. Uribe, Susanne Benseler, Kathleen M. O'Neil, Philip J. Hashkes, Gloria Higgins, Andrew S. Zeft, Daniel J. Lovell, Daniel J. Kingsbury, Anne Stevens, Deborah McCurdy, Peter Chira, Leslie Abramson, Thaschawee Arkachaisri, Sarah Campillo, Anne Eberhard, Aimee O. Hersh, Adam M. Huber, Susan Kim, Marisa Klein-GitelmanDeborah M. Levy, Suzanne C. Li, Thomas Mason, Esi Morgan DeWitt, Eyal Muscal, Lorien Nassi, Andreas Reiff, Kenneth Schikler, Nora G. Singer, Dawn Wahezi, Amy Woodward, Victor Espinosa, Jaime Guzman, Kristin Houghton, Peter Malleson, Ross Petty, Lori Tucker, Stuart Turvey, Elizabeth B. Brooks, Mary Lesko, Fatma Dedeoglu, Robert Fuhlbrigge, Melissa Hazen, Mary Beth Son, Robert Sundel, Diane Brown, Bracha Shaham, Ana Cabrera, Norman T. Ilowite, Raphael Hirsh, Daniel Kietz, Paul Rosen, Margalit Rosenkrank, Kathryn Torok, Lauren Pachman, Aisha Ali, Hermine Brunner, Thomas Griffin, Alexi Grom, Anne Johnson, Steven Spalding, Deborah Bork, Stacy Ardoin, Egla Rabinovich, Laura Schanberg, Rhonda Wilder, Bianca A. Lang, Suzanne Ramsey, Elizabeth Stringer, Aleasha Warner, Kathleen Haines, Yukiko Kimura, Jennifer Weiss, Imelda Balboni, Reuven Bromberg, Michal Cidon, Jennifer Frankovich, Dana Gerstbacher, Joyce J. Hsu, Tzielan Lee, Jane L. Park, Christy Sandborg, Steven Song, Ann Reed, Gaëlle Chédeville, Ciarán Duffy, Karen Duffy, Rosie Scuccimarri, Michele Gibbon, Andrew Eichenfield, Lisa Imundo, Candido Batres, Suzanne Bowyer, Susan Ballinger, Thomas Klausmeier, Andrea Hudgins, Helen Emery, Kristin Hayward, Christi Inman, Sarah Ringold, Elizabeth Shaw, Troy Torgerson, Jennifer Turner, Carol Wallace, Jennifer Wargula, Sarah Halford, Barry L. Myones, Marilynn Punaro, Virginia Pascual, Emily von Scheven, Adrienne Michels, Michael Henrickson, James Jarvis, John Bonsack, Sampath Prahalad

Research output: Contribution to journalArticle

112 Citations (Scopus)

Abstract

Objective. To compare the criteria for Wegener's granulomatosis (WG) of the American College of Rheumatology (ACR) with those of the European League Against Rheumatism/Pediatric Rheumatology European Society (EULAR/PRES) in a cohort of children with WG and other antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs), and to describe the interval to diagnosis, presenting features, and initial treatment for WG. Methods. Eligible patients had been diagnosed by site rheumatologists (termed the "MD diagnosis") since 2004. This diagnosis was used as a reference standard for sensitivity and specificity testing of the 2 WG classification criteria. Descriptive analyses were confined to ACR-classified WG patients. Results. MD diagnoses of 117 patients (82 of whom were female) were WG (n = 76), microscopic polyangiitis (n = 17), ANCA-positive pauci-immune glomerulonephritis (n = 5), Churg-Strauss syndrome (n = 2), and unclassified vasculitis (n = 17). The sensitivities of the ACR and EULAR/PRES classification criteria for WG among the spectrum of AAVs were 68.4% and 73.6%, respectively, and the specificities were 68.3% and 73.2%, respectively. Two more children were identified as having WG by the EULAR/PRES criteria than by the ACR criteria. For the 65 ACR-classified WG patients, the median age at diagnosis was 14.2 years (range 4-17 years), and the median interval from symptom onset to diagnosis was 2.7 months (range 0-49 months). The most frequent presenting features by organ system were constitutional (89.2%), pulmonary (80.0%), ear, nose, and throat (80.0%), and renal (75.4%). Fifty-four patients (83.1%) commenced treatment with the combination of corticosteroids and cyclophosphamide, with widely varying regimens; the remainder received methotrexate alone (n = 1), corticosteroids alone (n = 4), or a combination (n = 6). Conclusion. The EULAR/PRES criteria minimally improved diagnostic sensitivity and specificity for WG among a narrow spectrum of children with AAVs. Diagnostic delays may result from poor characterization of childhood WG. Initial therapy varied considerably among participating centers.

Original languageEnglish (US)
Pages (from-to)3413-3424
Number of pages12
JournalArthritis and Rheumatism
Volume60
Issue number11
DOIs
StatePublished - Nov 2009

Fingerprint

Granulomatosis with Polyangiitis
Rheumatology
Rheumatic Diseases
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
Therapeutics
Pediatrics
Antineutrophil Cytoplasmic Antibodies
Vasculitis
Adrenal Cortex Hormones
Microscopic Polyangiitis
Churg-Strauss Syndrome
Sensitivity and Specificity
Glomerulonephritis
Pharynx
Nose
Methotrexate
Cyclophosphamide
Ear

ASJC Scopus subject areas

  • Immunology
  • Immunology and Allergy
  • Rheumatology
  • Pharmacology (medical)

Cite this

Cabral, D. A., Uribe, A. G., Benseler, S., O'Neil, K. M., Hashkes, P. J., Higgins, G., ... Prahalad, S. (2009). Classification, presentation, and initial treatment of Wegener's granulomatosis in childhood. Arthritis and Rheumatism, 60(11), 3413-3424. https://doi.org/10.1002/art.24876

Classification, presentation, and initial treatment of Wegener's granulomatosis in childhood. / Cabral, David A.; Uribe, América G.; Benseler, Susanne; O'Neil, Kathleen M.; Hashkes, Philip J.; Higgins, Gloria; Zeft, Andrew S.; Lovell, Daniel J.; Kingsbury, Daniel J.; Stevens, Anne; McCurdy, Deborah; Chira, Peter; Abramson, Leslie; Arkachaisri, Thaschawee; Campillo, Sarah; Eberhard, Anne; Hersh, Aimee O.; Huber, Adam M.; Kim, Susan; Klein-Gitelman, Marisa; Levy, Deborah M.; Li, Suzanne C.; Mason, Thomas; DeWitt, Esi Morgan; Muscal, Eyal; Nassi, Lorien; Reiff, Andreas; Schikler, Kenneth; Singer, Nora G.; Wahezi, Dawn; Woodward, Amy; Espinosa, Victor; Guzman, Jaime; Houghton, Kristin; Malleson, Peter; Petty, Ross; Tucker, Lori; Turvey, Stuart; Brooks, Elizabeth B.; Lesko, Mary; Dedeoglu, Fatma; Fuhlbrigge, Robert; Hazen, Melissa; Son, Mary Beth; Sundel, Robert; Brown, Diane; Shaham, Bracha; Cabrera, Ana; Ilowite, Norman T.; Hirsh, Raphael; Kietz, Daniel; Rosen, Paul; Rosenkrank, Margalit; Torok, Kathryn; Pachman, Lauren; Ali, Aisha; Brunner, Hermine; Griffin, Thomas; Grom, Alexi; Johnson, Anne; Spalding, Steven; Bork, Deborah; Ardoin, Stacy; Rabinovich, Egla; Schanberg, Laura; Wilder, Rhonda; Lang, Bianca A.; Ramsey, Suzanne; Stringer, Elizabeth; Warner, Aleasha; Haines, Kathleen; Kimura, Yukiko; Weiss, Jennifer; Balboni, Imelda; Bromberg, Reuven; Cidon, Michal; Frankovich, Jennifer; Gerstbacher, Dana; Hsu, Joyce J.; Lee, Tzielan; Park, Jane L.; Sandborg, Christy; Song, Steven; Reed, Ann; Chédeville, Gaëlle; Duffy, Ciarán; Duffy, Karen; Scuccimarri, Rosie; Gibbon, Michele; Eichenfield, Andrew; Imundo, Lisa; Batres, Candido; Bowyer, Suzanne; Ballinger, Susan; Klausmeier, Thomas; Hudgins, Andrea; Emery, Helen; Hayward, Kristin; Inman, Christi; Ringold, Sarah; Shaw, Elizabeth; Torgerson, Troy; Turner, Jennifer; Wallace, Carol; Wargula, Jennifer; Halford, Sarah; Myones, Barry L.; Punaro, Marilynn; Pascual, Virginia; von Scheven, Emily; Michels, Adrienne; Henrickson, Michael; Jarvis, James; Bonsack, John; Prahalad, Sampath.

In: Arthritis and Rheumatism, Vol. 60, No. 11, 11.2009, p. 3413-3424.

Research output: Contribution to journalArticle

Cabral, DA, Uribe, AG, Benseler, S, O'Neil, KM, Hashkes, PJ, Higgins, G, Zeft, AS, Lovell, DJ, Kingsbury, DJ, Stevens, A, McCurdy, D, Chira, P, Abramson, L, Arkachaisri, T, Campillo, S, Eberhard, A, Hersh, AO, Huber, AM, Kim, S, Klein-Gitelman, M, Levy, DM, Li, SC, Mason, T, DeWitt, EM, Muscal, E, Nassi, L, Reiff, A, Schikler, K, Singer, NG, Wahezi, D, Woodward, A, Espinosa, V, Guzman, J, Houghton, K, Malleson, P, Petty, R, Tucker, L, Turvey, S, Brooks, EB, Lesko, M, Dedeoglu, F, Fuhlbrigge, R, Hazen, M, Son, MB, Sundel, R, Brown, D, Shaham, B, Cabrera, A, Ilowite, NT, Hirsh, R, Kietz, D, Rosen, P, Rosenkrank, M, Torok, K, Pachman, L, Ali, A, Brunner, H, Griffin, T, Grom, A, Johnson, A, Spalding, S, Bork, D, Ardoin, S, Rabinovich, E, Schanberg, L, Wilder, R, Lang, BA, Ramsey, S, Stringer, E, Warner, A, Haines, K, Kimura, Y, Weiss, J, Balboni, I, Bromberg, R, Cidon, M, Frankovich, J, Gerstbacher, D, Hsu, JJ, Lee, T, Park, JL, Sandborg, C, Song, S, Reed, A, Chédeville, G, Duffy, C, Duffy, K, Scuccimarri, R, Gibbon, M, Eichenfield, A, Imundo, L, Batres, C, Bowyer, S, Ballinger, S, Klausmeier, T, Hudgins, A, Emery, H, Hayward, K, Inman, C, Ringold, S, Shaw, E, Torgerson, T, Turner, J, Wallace, C, Wargula, J, Halford, S, Myones, BL, Punaro, M, Pascual, V, von Scheven, E, Michels, A, Henrickson, M, Jarvis, J, Bonsack, J & Prahalad, S 2009, 'Classification, presentation, and initial treatment of Wegener's granulomatosis in childhood', Arthritis and Rheumatism, vol. 60, no. 11, pp. 3413-3424. https://doi.org/10.1002/art.24876
Cabral DA, Uribe AG, Benseler S, O'Neil KM, Hashkes PJ, Higgins G et al. Classification, presentation, and initial treatment of Wegener's granulomatosis in childhood. Arthritis and Rheumatism. 2009 Nov;60(11):3413-3424. https://doi.org/10.1002/art.24876
Cabral, David A. ; Uribe, América G. ; Benseler, Susanne ; O'Neil, Kathleen M. ; Hashkes, Philip J. ; Higgins, Gloria ; Zeft, Andrew S. ; Lovell, Daniel J. ; Kingsbury, Daniel J. ; Stevens, Anne ; McCurdy, Deborah ; Chira, Peter ; Abramson, Leslie ; Arkachaisri, Thaschawee ; Campillo, Sarah ; Eberhard, Anne ; Hersh, Aimee O. ; Huber, Adam M. ; Kim, Susan ; Klein-Gitelman, Marisa ; Levy, Deborah M. ; Li, Suzanne C. ; Mason, Thomas ; DeWitt, Esi Morgan ; Muscal, Eyal ; Nassi, Lorien ; Reiff, Andreas ; Schikler, Kenneth ; Singer, Nora G. ; Wahezi, Dawn ; Woodward, Amy ; Espinosa, Victor ; Guzman, Jaime ; Houghton, Kristin ; Malleson, Peter ; Petty, Ross ; Tucker, Lori ; Turvey, Stuart ; Brooks, Elizabeth B. ; Lesko, Mary ; Dedeoglu, Fatma ; Fuhlbrigge, Robert ; Hazen, Melissa ; Son, Mary Beth ; Sundel, Robert ; Brown, Diane ; Shaham, Bracha ; Cabrera, Ana ; Ilowite, Norman T. ; Hirsh, Raphael ; Kietz, Daniel ; Rosen, Paul ; Rosenkrank, Margalit ; Torok, Kathryn ; Pachman, Lauren ; Ali, Aisha ; Brunner, Hermine ; Griffin, Thomas ; Grom, Alexi ; Johnson, Anne ; Spalding, Steven ; Bork, Deborah ; Ardoin, Stacy ; Rabinovich, Egla ; Schanberg, Laura ; Wilder, Rhonda ; Lang, Bianca A. ; Ramsey, Suzanne ; Stringer, Elizabeth ; Warner, Aleasha ; Haines, Kathleen ; Kimura, Yukiko ; Weiss, Jennifer ; Balboni, Imelda ; Bromberg, Reuven ; Cidon, Michal ; Frankovich, Jennifer ; Gerstbacher, Dana ; Hsu, Joyce J. ; Lee, Tzielan ; Park, Jane L. ; Sandborg, Christy ; Song, Steven ; Reed, Ann ; Chédeville, Gaëlle ; Duffy, Ciarán ; Duffy, Karen ; Scuccimarri, Rosie ; Gibbon, Michele ; Eichenfield, Andrew ; Imundo, Lisa ; Batres, Candido ; Bowyer, Suzanne ; Ballinger, Susan ; Klausmeier, Thomas ; Hudgins, Andrea ; Emery, Helen ; Hayward, Kristin ; Inman, Christi ; Ringold, Sarah ; Shaw, Elizabeth ; Torgerson, Troy ; Turner, Jennifer ; Wallace, Carol ; Wargula, Jennifer ; Halford, Sarah ; Myones, Barry L. ; Punaro, Marilynn ; Pascual, Virginia ; von Scheven, Emily ; Michels, Adrienne ; Henrickson, Michael ; Jarvis, James ; Bonsack, John ; Prahalad, Sampath. / Classification, presentation, and initial treatment of Wegener's granulomatosis in childhood. In: Arthritis and Rheumatism. 2009 ; Vol. 60, No. 11. pp. 3413-3424.
@article{91f106efe1b042abbae442fef8a4700c,
title = "Classification, presentation, and initial treatment of Wegener's granulomatosis in childhood",
abstract = "Objective. To compare the criteria for Wegener's granulomatosis (WG) of the American College of Rheumatology (ACR) with those of the European League Against Rheumatism/Pediatric Rheumatology European Society (EULAR/PRES) in a cohort of children with WG and other antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs), and to describe the interval to diagnosis, presenting features, and initial treatment for WG. Methods. Eligible patients had been diagnosed by site rheumatologists (termed the {"}MD diagnosis{"}) since 2004. This diagnosis was used as a reference standard for sensitivity and specificity testing of the 2 WG classification criteria. Descriptive analyses were confined to ACR-classified WG patients. Results. MD diagnoses of 117 patients (82 of whom were female) were WG (n = 76), microscopic polyangiitis (n = 17), ANCA-positive pauci-immune glomerulonephritis (n = 5), Churg-Strauss syndrome (n = 2), and unclassified vasculitis (n = 17). The sensitivities of the ACR and EULAR/PRES classification criteria for WG among the spectrum of AAVs were 68.4{\%} and 73.6{\%}, respectively, and the specificities were 68.3{\%} and 73.2{\%}, respectively. Two more children were identified as having WG by the EULAR/PRES criteria than by the ACR criteria. For the 65 ACR-classified WG patients, the median age at diagnosis was 14.2 years (range 4-17 years), and the median interval from symptom onset to diagnosis was 2.7 months (range 0-49 months). The most frequent presenting features by organ system were constitutional (89.2{\%}), pulmonary (80.0{\%}), ear, nose, and throat (80.0{\%}), and renal (75.4{\%}). Fifty-four patients (83.1{\%}) commenced treatment with the combination of corticosteroids and cyclophosphamide, with widely varying regimens; the remainder received methotrexate alone (n = 1), corticosteroids alone (n = 4), or a combination (n = 6). Conclusion. The EULAR/PRES criteria minimally improved diagnostic sensitivity and specificity for WG among a narrow spectrum of children with AAVs. Diagnostic delays may result from poor characterization of childhood WG. Initial therapy varied considerably among participating centers.",
author = "Cabral, {David A.} and Uribe, {Am{\'e}rica G.} and Susanne Benseler and O'Neil, {Kathleen M.} and Hashkes, {Philip J.} and Gloria Higgins and Zeft, {Andrew S.} and Lovell, {Daniel J.} and Kingsbury, {Daniel J.} and Anne Stevens and Deborah McCurdy and Peter Chira and Leslie Abramson and Thaschawee Arkachaisri and Sarah Campillo and Anne Eberhard and Hersh, {Aimee O.} and Huber, {Adam M.} and Susan Kim and Marisa Klein-Gitelman and Levy, {Deborah M.} and Li, {Suzanne C.} and Thomas Mason and DeWitt, {Esi Morgan} and Eyal Muscal and Lorien Nassi and Andreas Reiff and Kenneth Schikler and Singer, {Nora G.} and Dawn Wahezi and Amy Woodward and Victor Espinosa and Jaime Guzman and Kristin Houghton and Peter Malleson and Ross Petty and Lori Tucker and Stuart Turvey and Brooks, {Elizabeth B.} and Mary Lesko and Fatma Dedeoglu and Robert Fuhlbrigge and Melissa Hazen and Son, {Mary Beth} and Robert Sundel and Diane Brown and Bracha Shaham and Ana Cabrera and Ilowite, {Norman T.} and Raphael Hirsh and Daniel Kietz and Paul Rosen and Margalit Rosenkrank and Kathryn Torok and Lauren Pachman and Aisha Ali and Hermine Brunner and Thomas Griffin and Alexi Grom and Anne Johnson and Steven Spalding and Deborah Bork and Stacy Ardoin and Egla Rabinovich and Laura Schanberg and Rhonda Wilder and Lang, {Bianca A.} and Suzanne Ramsey and Elizabeth Stringer and Aleasha Warner and Kathleen Haines and Yukiko Kimura and Jennifer Weiss and Imelda Balboni and Reuven Bromberg and Michal Cidon and Jennifer Frankovich and Dana Gerstbacher and Hsu, {Joyce J.} and Tzielan Lee and Park, {Jane L.} and Christy Sandborg and Steven Song and Ann Reed and Ga{\"e}lle Ch{\'e}deville and Ciar{\'a}n Duffy and Karen Duffy and Rosie Scuccimarri and Michele Gibbon and Andrew Eichenfield and Lisa Imundo and Candido Batres and Suzanne Bowyer and Susan Ballinger and Thomas Klausmeier and Andrea Hudgins and Helen Emery and Kristin Hayward and Christi Inman and Sarah Ringold and Elizabeth Shaw and Troy Torgerson and Jennifer Turner and Carol Wallace and Jennifer Wargula and Sarah Halford and Myones, {Barry L.} and Marilynn Punaro and Virginia Pascual and {von Scheven}, Emily and Adrienne Michels and Michael Henrickson and James Jarvis and John Bonsack and Sampath Prahalad",
year = "2009",
month = "11",
doi = "10.1002/art.24876",
language = "English (US)",
volume = "60",
pages = "3413--3424",
journal = "Arthritis and Rheumatology",
issn = "2326-5191",
publisher = "John Wiley and Sons Ltd",
number = "11",

}

TY - JOUR

T1 - Classification, presentation, and initial treatment of Wegener's granulomatosis in childhood

AU - Cabral, David A.

AU - Uribe, América G.

AU - Benseler, Susanne

AU - O'Neil, Kathleen M.

AU - Hashkes, Philip J.

AU - Higgins, Gloria

AU - Zeft, Andrew S.

AU - Lovell, Daniel J.

AU - Kingsbury, Daniel J.

AU - Stevens, Anne

AU - McCurdy, Deborah

AU - Chira, Peter

AU - Abramson, Leslie

AU - Arkachaisri, Thaschawee

AU - Campillo, Sarah

AU - Eberhard, Anne

AU - Hersh, Aimee O.

AU - Huber, Adam M.

AU - Kim, Susan

AU - Klein-Gitelman, Marisa

AU - Levy, Deborah M.

AU - Li, Suzanne C.

AU - Mason, Thomas

AU - DeWitt, Esi Morgan

AU - Muscal, Eyal

AU - Nassi, Lorien

AU - Reiff, Andreas

AU - Schikler, Kenneth

AU - Singer, Nora G.

AU - Wahezi, Dawn

AU - Woodward, Amy

AU - Espinosa, Victor

AU - Guzman, Jaime

AU - Houghton, Kristin

AU - Malleson, Peter

AU - Petty, Ross

AU - Tucker, Lori

AU - Turvey, Stuart

AU - Brooks, Elizabeth B.

AU - Lesko, Mary

AU - Dedeoglu, Fatma

AU - Fuhlbrigge, Robert

AU - Hazen, Melissa

AU - Son, Mary Beth

AU - Sundel, Robert

AU - Brown, Diane

AU - Shaham, Bracha

AU - Cabrera, Ana

AU - Ilowite, Norman T.

AU - Hirsh, Raphael

AU - Kietz, Daniel

AU - Rosen, Paul

AU - Rosenkrank, Margalit

AU - Torok, Kathryn

AU - Pachman, Lauren

AU - Ali, Aisha

AU - Brunner, Hermine

AU - Griffin, Thomas

AU - Grom, Alexi

AU - Johnson, Anne

AU - Spalding, Steven

AU - Bork, Deborah

AU - Ardoin, Stacy

AU - Rabinovich, Egla

AU - Schanberg, Laura

AU - Wilder, Rhonda

AU - Lang, Bianca A.

AU - Ramsey, Suzanne

AU - Stringer, Elizabeth

AU - Warner, Aleasha

AU - Haines, Kathleen

AU - Kimura, Yukiko

AU - Weiss, Jennifer

AU - Balboni, Imelda

AU - Bromberg, Reuven

AU - Cidon, Michal

AU - Frankovich, Jennifer

AU - Gerstbacher, Dana

AU - Hsu, Joyce J.

AU - Lee, Tzielan

AU - Park, Jane L.

AU - Sandborg, Christy

AU - Song, Steven

AU - Reed, Ann

AU - Chédeville, Gaëlle

AU - Duffy, Ciarán

AU - Duffy, Karen

AU - Scuccimarri, Rosie

AU - Gibbon, Michele

AU - Eichenfield, Andrew

AU - Imundo, Lisa

AU - Batres, Candido

AU - Bowyer, Suzanne

AU - Ballinger, Susan

AU - Klausmeier, Thomas

AU - Hudgins, Andrea

AU - Emery, Helen

AU - Hayward, Kristin

AU - Inman, Christi

AU - Ringold, Sarah

AU - Shaw, Elizabeth

AU - Torgerson, Troy

AU - Turner, Jennifer

AU - Wallace, Carol

AU - Wargula, Jennifer

AU - Halford, Sarah

AU - Myones, Barry L.

AU - Punaro, Marilynn

AU - Pascual, Virginia

AU - von Scheven, Emily

AU - Michels, Adrienne

AU - Henrickson, Michael

AU - Jarvis, James

AU - Bonsack, John

AU - Prahalad, Sampath

PY - 2009/11

Y1 - 2009/11

N2 - Objective. To compare the criteria for Wegener's granulomatosis (WG) of the American College of Rheumatology (ACR) with those of the European League Against Rheumatism/Pediatric Rheumatology European Society (EULAR/PRES) in a cohort of children with WG and other antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs), and to describe the interval to diagnosis, presenting features, and initial treatment for WG. Methods. Eligible patients had been diagnosed by site rheumatologists (termed the "MD diagnosis") since 2004. This diagnosis was used as a reference standard for sensitivity and specificity testing of the 2 WG classification criteria. Descriptive analyses were confined to ACR-classified WG patients. Results. MD diagnoses of 117 patients (82 of whom were female) were WG (n = 76), microscopic polyangiitis (n = 17), ANCA-positive pauci-immune glomerulonephritis (n = 5), Churg-Strauss syndrome (n = 2), and unclassified vasculitis (n = 17). The sensitivities of the ACR and EULAR/PRES classification criteria for WG among the spectrum of AAVs were 68.4% and 73.6%, respectively, and the specificities were 68.3% and 73.2%, respectively. Two more children were identified as having WG by the EULAR/PRES criteria than by the ACR criteria. For the 65 ACR-classified WG patients, the median age at diagnosis was 14.2 years (range 4-17 years), and the median interval from symptom onset to diagnosis was 2.7 months (range 0-49 months). The most frequent presenting features by organ system were constitutional (89.2%), pulmonary (80.0%), ear, nose, and throat (80.0%), and renal (75.4%). Fifty-four patients (83.1%) commenced treatment with the combination of corticosteroids and cyclophosphamide, with widely varying regimens; the remainder received methotrexate alone (n = 1), corticosteroids alone (n = 4), or a combination (n = 6). Conclusion. The EULAR/PRES criteria minimally improved diagnostic sensitivity and specificity for WG among a narrow spectrum of children with AAVs. Diagnostic delays may result from poor characterization of childhood WG. Initial therapy varied considerably among participating centers.

AB - Objective. To compare the criteria for Wegener's granulomatosis (WG) of the American College of Rheumatology (ACR) with those of the European League Against Rheumatism/Pediatric Rheumatology European Society (EULAR/PRES) in a cohort of children with WG and other antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs), and to describe the interval to diagnosis, presenting features, and initial treatment for WG. Methods. Eligible patients had been diagnosed by site rheumatologists (termed the "MD diagnosis") since 2004. This diagnosis was used as a reference standard for sensitivity and specificity testing of the 2 WG classification criteria. Descriptive analyses were confined to ACR-classified WG patients. Results. MD diagnoses of 117 patients (82 of whom were female) were WG (n = 76), microscopic polyangiitis (n = 17), ANCA-positive pauci-immune glomerulonephritis (n = 5), Churg-Strauss syndrome (n = 2), and unclassified vasculitis (n = 17). The sensitivities of the ACR and EULAR/PRES classification criteria for WG among the spectrum of AAVs were 68.4% and 73.6%, respectively, and the specificities were 68.3% and 73.2%, respectively. Two more children were identified as having WG by the EULAR/PRES criteria than by the ACR criteria. For the 65 ACR-classified WG patients, the median age at diagnosis was 14.2 years (range 4-17 years), and the median interval from symptom onset to diagnosis was 2.7 months (range 0-49 months). The most frequent presenting features by organ system were constitutional (89.2%), pulmonary (80.0%), ear, nose, and throat (80.0%), and renal (75.4%). Fifty-four patients (83.1%) commenced treatment with the combination of corticosteroids and cyclophosphamide, with widely varying regimens; the remainder received methotrexate alone (n = 1), corticosteroids alone (n = 4), or a combination (n = 6). Conclusion. The EULAR/PRES criteria minimally improved diagnostic sensitivity and specificity for WG among a narrow spectrum of children with AAVs. Diagnostic delays may result from poor characterization of childhood WG. Initial therapy varied considerably among participating centers.

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